Résumé
Malgré les progrès de la réanimation, la mortalité néonatale des enfants porteurs de HCD reste élevée, proche de 30–40 %, essentiellement du fait de l’hypoplasie pulmonaire et d’une hypertension artérielle pulmonaire. Les principales séquelles observées sont respiratoires (HTAP chronique, dysplasie bronchopulmonaire, susceptibilité aux infections virales), digestives (reflux gastro-oesophagien, trouble de l’oralité), nutritionnelles (dénutrition d’origine multifactorielle: RGO, trouble de l’oralité, insuffisance respiratoire) et orthopédiques (scoliose). La prise en charge actuelle de ces complications est tardive: elle nécessite une intervention plus précoce, qui doit débuter dès la période néonatale. Les objectifs de la prise en charge médicale seront: 1) d’assurer une oxygénation tissulaire et la décarboxylation tout en minimisant le baro-volotraumatisme du poumon; 2) d’assurer une fonction circulatoire adéquate en limitant les conséquences de l’HTAP; 3) de prévenir la morbidité respiratoire et digestive. Elle nécessite impérativement un suivi et une prise en charge multidisciplinaire spécialisée, du fait de l’intrication de ces différentes complications.
Abstract
Despite improvements in intensive care, lung hypoplasia and persistent pulmonary hypertension cause a high mortality rate in newborns with CDH. Sequelae including bronchopulmonary dysplasia, chronic pulmonary hypertension, gastro-oesophageal reflux, growth retardation and oral aversion are observed in half the surviving infants. The aims of post-natal management are: 1) to provide adequate tissular oxygenation and decarboxylation, with minimal lung volo-or barotrauma; 2) to optimise circulatory function through PPHN management; 3) to prevent respiratory and nutritional morbidity. Long-term follow-up and multidisciplinary management are required.
Références
Skari H, Bjornland K, Haugen G, et al (2000) Congenital diaphragmatic hernia: a meta-analysis of mortality factors. J Pediatr Surg 35:1187–1197
Slavotinek AM (2004) Fryns syndrome: a review of the phenotype and diagnostic guidelines. Am J Med Genet A 124:427–433
Enns GM, Cox VA, Goldstein RB, et al (1998) Congenital diaphragmatic defects and associated syndromes, malformations, and chromosome anomalies: a retrospective study of 60 patients and literature review. Am J Med Genet 79:215–225
Stege G, Fenton A, Jaffray B (2003) Nihilism in the 1990s: the true mortality of congenital diaphragmatic hernia. Pediatrics 112:532–535
Jaillard S, Pierrat V, Dubois A, et al (2003) Outcome at 2 years of infants with congenital diaphragmatic hernia: a populationbased study. Ann Thorac Surg 75:250–256
Rudolph A, Heymann MA (1970) Circulation changes during growth in the fetal lamb. Circ Res 26:289–299
Abman SH, Chatfield BA, Hall SL, McMurtry IF (1990) Role of endothelium-derived relaxing factor during transition of pulmonary circulation at birth. Am J Physiol 259:H1921–H1927
Storme L, Rairigh RL, Parker YA, et al (1999) Potassium channel blockade inhibits shear stress-induced pulmonary vasodilation in the ovine fetus. Am J Physiol 276:L220–L228
Magnenant E, Jaillard S, Deruelle P, et al (2003) Role of the alpha2-adrenoceptors on the pulmonary circulation in the ovine fetus. Pediatr Res 54:44–51
Houfflin-Debarge V, Deruelle P, Jaillard S, et al (2005) Effects of antenatal glucocorticoids on circulatory adaptation at birth in the ovine fetus. Biol Neonate 88:73–78
Bargy F, Beaudoin S, Barbet P (2006) Fetal lung growth in congenital diaphragmatic hernia. Fetal Diagn Ther 21:39–44
Boucherat O, Benachi A, Barlier-Mur AM, et al (2007) Decreased lung fibroblast growth factor 18 and elastin in human congenital diaphragmatic hernia and animal models. Am J Respir Crit Care Med 175:1066–1077
Tenbrinck R, Gaillard JL, Tibboel D, et al (1992) Pulmonary vascular abnormalities in experimentally induced congenital diaphragmatic hernia in rats. J Pediatr Surg 27:862–865
Shehata SM, Tibboel D, Sharma HS, Mooi WJ (1999) Impaired structural remodelling of pulmonary arteries in newborns with congenital diaphragmatic hernia: a histological study of 29 cases. J Pathol 189:112–118
Walsh M, Stork E (2001) Persistent pulmonary hypertension of the newborn. Rational therapy bases on pathophysiology. Clin Perinatol 28:609–627
Vukcevic Z, Coppola CP, Hults C, Gosche JR (2005) Nitrovasodilator responses in pulmonary arterioles from rats with nitrofen-induced congenital diaphragmatic hernia. J Pediatr Surg 40:1706–1711
Newell MA, Au-Fliegner M, Coppola CP, Gosche JR (1998) Hypoxic pulmonary vasoconstriction is impaired in rats with nitrofen-induced congenital diaphragmatic hernia. J Pediatr Surg 33:1358–1362
Solari V, Piotrowska AP, Puri P (2003) Expression of heme oxygenase-1 and endothelial nitric oxide synthase in the lung of newborns with congenital diaphragmatic hernia and persistent pulmonary hypertension. J Pediatr Surg 38:808–813
Shehata SM, Sharma HS, Mooi WJ, Tibboel D (2006) Pulmonary hypertension in human newborns with congenital diaphragmatic hernia is associated with decreased vascular expression of nitric-oxide synthase. Cell Biochem Biophys 44:147–155
de Lagausie P, de Buys-Roessingh A, Ferkdadji L, et al (2005) Endothelin receptor expression in human lungs of newborns with congenital diaphragmatic hernia. J Pathol 205:112–118
Tourneux P, Rakza T, Bouissou A, et al (2008) Pulmonary circulatory effects of norepinephrine in newborn infants with persistent pulmonary hypertension (PPHN). J Pediatr, in press
Folkesson HG, Chapin CJ, Beard LL, et al (2006) Congenital diaphragmatic hernia prevents absorption of distal air space fluid in late-gestation rat fetuses. Am J Physiol Lung Cell Mol Physiol 290: L478–L484
Schwartz SM, Vermilion RP, Hirschl RB (1994) Evaluation of left ventricular mass in children with left-sided congenital diaphragmatic hernia. J Pediatr 125:447–451
Karamanoukian HL, O’Toole SJ, Rossman JR, et al (1996) Can cardiac weight predict lung weight in patients with congenital diaphragmatic hernia? J Pediatr Surg 31:823–825
Karamanoukian HL, Glick PL, Wilcox DT, et al (1995) Pathophysiology of congenital diaphragmatic hernia. XI: anatomic and biochemical characterization of the heart in the fetal lamb CDH model, J Pediatr Surg 30:925–929
Takayasu H, Sato H, Sugimoto K, Puri P (2008) Downregulation of GATA4 and GATA6 in the heart of rats with nitrofen-induced diaphragmatic hernia. J Pediatr Surg 43:362–366
Guarino N, Puri P (2002) Antenatal dexamethasone enhances endothelin-1 synthesis and gene expression in the heart in congenital diaphragmatic hernia in rats. J Pediatr Surg 37:1563–1567
Azarow K, Messineo A, Pearl R, et al (1997) Congenital diaphragmatic hernia: a tale of two cities: the Toronto experience. J Pediatr Surg 32:395–400
Bohn D (2002) Congenital diaphragmatic hernia. Am J Respir Crit Care Med 166:911–915
Downard C, Jaksic T, Garza J, et al (2003) Analysis of an improved survival rate for congenital diaphragmatic hernia. J Pediatr Surg 38:729–732
Boloker J, Bateman D, Wung JT, Stolar C (2002) Congenital diaphragmatic hernia in 120 infants treated consecutively with permissive hypercapnia/spontaneous respiration/elective repair. J Pediatr Surg 37:357–366
Finer NN, Tierney A, Etches PC, et al (1998) Congenital diaphragmatic hernia: developing a protocolized approach. J Pediatr Surg 33:1331–1337
Sakurai Y, Azarow K, Cutz E, et al (1999) Pulmonary barotrauma in congenital diaphragmatic hernia: a clinicopathological correlation. J Pediatr Surg. 34(12):1813–1817
Desfrere L, Jarreau PH, Dommergues M, et al (2000) Impact of delayed repair and elective high-frequency oscillatory ventilation on survival of antenatally diagnosed congenital diaphragmatic hernia: first application of these strategies in the more ’severe’ subgroup of antenatally diagnosed newborns. Intensive Care Med 26:934–934
Reyes C, Chang LK, Waffarn F, et al (1998) Delayed repair of congenital diaphragmatic hernia with early high-frequency oscillatory ventilation during preoperative stabilization. J Pediatr Surg 33:1010–1014
Boucherat O, Benachi A, Chailley-Heu B, et al (2004) Surfactant maturation is not delayed in human fetuses with diaphragmatic hernia. PLoS Med 4:e237
Lally KP, Lally PA, Langham MR, et al (2004) Surfactant does not improve survival rate in preterm infants with congenital diaphragmatic hernia. J Pediatr Surg 39:829–833
Suda K, Bigras JL, Bohn D, et al (2000) Echocardiographic predictors of outcome in newborns with congenital diaphragmatic hernia. Pediatrics 105:1106–1109
Bohn D (2002) Congenital diaphragmatic hernia. Am J Respir Crit Care Med 166:911–915
Berman RE, Barst RJ (2006) Pulmonary arterial hypertension: a comprehensive review of pharmacological treatment. Treat Respir Med 5:117–127
Chiu P, HL Hedrick (2008) Postnatal management and long-term outcome for survivors with congenital diaphragmatic hernia. Prenat Diagn 28:592–603
Inamura N, A Kubota, T Nakajima, et al (2005) A proposal of new therapeutic strategy for antenatally diagnosed congenital diaphragmatic hernia. J Ped Surg 40:1315–1319
Houfflin Debarge V, Sicot B, Jaillard S, et al (2007) Mechanisms of pain-induced pulmonary vasoconstriction: experimental study in fetal lambs. Anesth Analg 104:799–806
Jaillard S, Houfflin-Debarge V, Riou Y, et al (2001) Effects of catecholamines on the pulmonary circulation in the ovine fetus. Am J Physiol 281:R607–R614
Liet JM, Boscher C, Gras-Leguen C, et al (2002) Dopamine effects on pulmonary artery pressure in hypotensive preterm infants with patent ductus arteriosus J Pediatr 140:373–375
Bouissou A, Rakza T, Klosowski S, et al (2008) Hypotension in preterm infants with significant patent ductus arteriosus: effects of dopamine. J Pediatr, in press
Houfflin-Debarge V, Delelis A, Jaillard S, et al (2005) Effects of nociceptive stimuli on the pulmonary circulation in the ovine fetus. Am J Physiol Regul Integr Comp Physiol 288:R547–R553
Nishina H, Ozaki T, Hanson MA, Poston L (1999) Mechanisms of noradrenaline-induced vasorelaxation in isolated femoral arteries of the neonatal rat. Br J Pharmacol 127:809–812
Tourneux P, Rakza T, Abazine A, et al (2008) Noradrenaline for management of septic shock refractory to fluid loading and dopamine or dobutamine in full-term newborn infants. Acta Paediatr 97:177–180
Dellinger RP, Carlet JM, Masur H, et al (2004) Surviving sepsis campaign guidelines for management of severe sepsis and septic shock. Crit Care Med 32:858–873
Jaillard S, Houfflin-Debarge V, Riou Y, et al (2001) Effects of catecholamines on the pulmonary circulation in the ovine fetus. Am J Physiol 281: R607–614
Jaillard S, Elbaz F, Bresson-Just S, et al (2004) Pulmonary vasodilator effects of norepinephrine during the development of chronic pulmonary hypertension in neonatal lambs. Br J Anaesth 93:818–824
Deruelle P, Houfflin-Debarge V, Magnenant E, et al (2003) Effects of antenatal glucocorticoids on pulmonary vascular reactivity in the ovine fetus. Am J Obstet Gynecol 189:208–215
Jaillard S, Houfflin-Debarge V, Storme L (2003) Higher risk of persistent pulmonary hypertension of the newborn after caesarean section. J Perinat Med 31:538–539
Finer NN, Barrington KJ (2006) Nitric oxide for respiratory failure in infants born at or near term. Cochrane Database System Rev (4):CD000399
Archer SL, Huang JM, Hampl V, et al (1994) Nitric oxide and cGMP cause vasorelaxation by activation of a charybdotoxinsensitive K channel by cGMP-dependent protein kinase. Proc Natl Acad Sci USA 91:7583–7587
Larrue B, Jaillard S, Lorthioir M, et al (2005) Pulmonary vascular effects of sildenafil on the development of chronic pulmonary hypertension in the ovine fetus. Am J Physiol Lung Cell Mol Physiol 288:L1193–L2000
Kinsella JP, Ivy DD, Abman SH (2005) Pulmonary vasodilator therapy in congenital diaphragmatic hernia: acute, late, and chronic pulmonary hypertension. Semin Perinatol 29:123–128
The Neonatal Inhaled Nitric Oxide Study Group (NINOS) (1997) Inhaled nitric oxide and hypoxic respiratory failure in infants with congenital diaphragmatic hernia. Pediatrics 99:838–845
Kinsella JP (2008) Inhaled NO in term newborns. Early Human Dev 84:709–716
Larrue B, Jaillard S, Lorthioir M, et al (2005) Pulmonary vascular effects of sildenafil on the development of chronic pulmonary hypertension in the ovine fetus. Am J Physiol Lung Cell Mol Physiol 288: L1193–L2000
Dillon PW, Cilley RE, Hudome SM, et al (1995) Nitric oxide reversal of recurrent pulmonary hypertension and respiratory failure in an infant with CDH after successful ECMO therapy. J Pediatr Surg 30:743–744
Kinsella JP, Parker TA, Ivy DD, Abman SH (2003) Noninvasive delivery of inhaled nitric oxide therapy for late pulmonary hypertension in newborn infants with congenital diaphragmatic hernia. J Pediatr 142:397–401
Sebkhi A, Strange JW, Phillips SC, et al (2003) Phosphodiesterase type 5 as a target for the treatment of hypoxia-induced pulmonary hypertension. Circulation 107:3230–3235
Noori S, Friedlich P, Wong P, et al (2007) Cardiovascular effects of sildenafil in neonates and infants with congenital diaphragmatic hernia and pulmonary hypertension. Neonatology 91:92–100
Keller RL, Moore P, Teitel D, et al (2006) Abnormal vascular tone in infants and children with lung hypoplasia: findings from cardiac catheterization and the response to chronic therapy. Pediatr Crit Care Med 7:589–594
Bétrémieux P, Gaillot T, de la Pintière A, et al (2004) Congenital diaphragmatic hernia: prenatal diagnosis permits immediate intensive care with high survival rate in isolated cases. A population-based study. Prenat Diagn 24:487–493
Ivy D, Le Cras T, Horan M, Abman S (1998) Increased lung preproET-1 and decreased ETB-receptor gene expression in fetal pulmonary hypertension. Am J Physiol 274:L535–L541
Kavanagh M, Battistini B, Jean S, et al (2001) Effect of ABT-627 (A-147627), a potent selective ET(A) receptor antagonist, on the cardiopulmonary profile of newborn lambs with surgically-induced diaphragmatic hernia. Br J Pharmacol 134:1679–1688
Thébaud B, de Lagausie P, Forgues D, et al (2000) ET(A)-receptor blockade and ET(B)-receptor stimulation in experimental congenital diaphragmatic hernia. Am J Physiol Lung Cell Mol Physiol 278:L923–L933
Chiu P, Hedrick HL (2008) Postnatal management and long-term outcome for survivors with congenital diaphragmatic hernia. Prenat Diagn 28:592–603
UK Collaborative ECMO Group (1998) The collaborative UK ECMO trial: follow-up to 1 year of age. 101:E1
El Hassani A, Michaud L, Chartier A, et al (2005) Cow’s milk protein allergy after neonatal intestinal surgery. Arch Pediatr 12(2):134–139
Bohn D (2002) Congenital diaphragmatic hernia. Am J Respir Crit Care Med 166:911–915
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Storme, L., Rakza, T., Sfeir, R. et al. Prise en charge médicale per- et postnatale de la hernie congénitale diaphragmatique. Rev. med. perinat. 1, 26–36 (2009). https://doi.org/10.1007/s12611-009-0008-5
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DOI: https://doi.org/10.1007/s12611-009-0008-5